Neurology Flashcards
Diagnostic criteria for encephalitis.
Major Criteria - acute onset of behavioural disturbance <24 hours
Minor Criteria
- Documented fever
- Seizures not related to pre-existing disorder
- New onset focal neurology
- CSF WBC >5
- Brain imaging changes
- EEG changes
What activates NMDA receptors?
Glutamate
Which part of the internal capsule does the corticospinal tract run through?
Posterior limb
When are electrophysiologic studies useful?
Peripheral neuropathy
Entrapment neuropathy
Radiculopathies
Muscle disorders
What are the expected EMG findings in neuropathy?
Denervation
Fibrillations
Fasciculations
Positive sharp waves
What are the expected EMG findings in myopathies?
Decreased amplitudes
Small polyphasic MUAPs
What is the most sensitive test for myasthenia gravis?
Single fibre EMG
What is the argyle Robertson pupil? And what is the differential diagnosis?
Bilateral small pupils that accomodate but do not react.
Neurosyphilis
Diabetic neuropathy
What is the diagnostic criteria for MELAS?
- Stroke like episodes before 40
- Encephalopathy (seizures or dementia)
- Lactic acidosis OR “ragged red fibres” on skeletal muscle biopsy
What is the treatment of MELAS patients with an acute stroke like episode?
Intravenous followed by oral arginine hydrochloride
What are the 2 core clinical criteria for MMN?
- Stepwise progressive asymmetric limb weakness for more than a month
- No objective sensory change (apart from mild vibration sense changes)
What are the exclusion criteria for MMN?
Upper motor neuron signs
Marked bulbar involvement
Sensory impairment
Diffuse symmetrical weakness at the start
What is the treatment of MMN?
IVIG
Not responsive to plasma exchange or steroids
What is the classical presentation of CJD? And what is the median survival?
Rapidly progressive dementia with myoclonus. Median survival 4-5 months.
What is variant CJD and what is the prognosis?
Bovine to human transmission of CJD resulting in similar clinical phenotype (dementia and myoclonus)
Median survival 11 months
Type 1 myotonic dystrophy results from an expansion of which trinucleotide repeat?
CTG
How does having a malignancy influence the prognosis of NMDA encephalitis?
Improved prognosis with malignancy.
What protein is associated with duchennes muscular dystrophy?
Dystrophin
What is the ABCD2 score?
Age - >60 BP - >140/90 Clinical - unilateral weak (2), speech (1) Duration - 10-59mins (1), >60 (2) Diabetes - 1
What are the common residual deficits after Wernickes?
- Horizontal nystagmus (60%)
- Ataxia (60%)
- Memory deficit (80%)
What is the treatment of Ramsay Hunt Syndrome?
Valacyclovir 10 days
Prednisone 5 days
What lesion causes Alexia without agraphia?
Dominant PCA infarction
Describe fragile X syndrome.
X linked dominant condition from CGG trinucleotide repeats (>200) (FMR1) which results in intellectual disability, ataxia, tremor and Parkinsonism.
Type 1 muscle fibres description.
Red colour High mitochondria Slow twitch fibre Slow conduction velocity Fatigue resistant Aerobic metabolism
Where is Broca’s area?
Inferior frontal gyrus
What are the features of a 4th nerve palsy?
Vertical diplopia
Eyes that turn up and out
Anti Hu antibody
Disease association?
Presentation?
Small cell lung cancer
Encephalomyelitis or cerebellar Syndrome
Anti Yo antibodies
Disease association?
Presentation?
Ovarian and breast cancer
Cerebellar Syndrome
Anti GAD antibodies
Disease association?
Presentation?
Small cell/ breast/ colorectal cancer
Stiff person syndrome
Anti Ri antibodies
Disease association
Presentation?
Small cell and breast cancer
Ocular opsoclonus myoclonus
Mechanism of riluzole?
Unknown really but
?sodium channel blockade
?Glutamate antagonist
What features of Parkinson’s are uncommon in drug induced Parkinsonism?
Rigidity and rest tremor
How does phenytoin cause anemia?
Folate deficiency
What medication is first line in the prevention of cluster headaches?
Verapamil
What percentage of children with absence seizures will be free of seizures by 15 years?
95%
Wernickes aphasia Type of aphasia? Lesion location? Fluent or non fluent? Comprehension normal or abnormal? Repetition normal or impaired?
Receptive aphasia Superior temporal gyrus Fluent speech Abnormal comprehension Normal repetition
Conduction aphasia Type of aphasia? Lesion location? Fluent or non fluent? Comprehension normal or abnormal? Repetition normal or impaired?
Dissociative aphasia Actuate fasiculus Fluent aphasia Abnormal comprehension Poor repetition
Brocas aphasia Type of aphasia? Lesion location? Fluent or non fluent? Comprehension normal or abnormal? Repetition normal or impaired?
Expressive dysphasia Inferior frontal gyrus Non fluent speech Normal comprehension Poor repetition
A lesion of which artery results in a lateral medullary syndrome?
PICA
What is the Wallenberg Syndrome?
Lateral medullary syndrome from PICA stroke
Vertigo
Ipsilateral horners
Ipsilateral bulbar muscle weakness
Loss of pain and temperature on contralateral side
What is Dejerine Syndrome?
Small perforating branch occlusion
Contralateral Hemiplegia
Contralateral sensory loss
Contralateral limb ataxia
What are the features of a AICA infarct?
Vertigo
Ataxia
Facial palsy
Hypoacusis
What are the features of myotonic dystrophy?
Distal weakness
Dominant (autosomal)
Diabetes
Dysarthria
